Information about caring for special needs & medically fragile children.

Archive for March, 2011

Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes inside and outside the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia, bile ducts that are located inside or outside the liver are blocked. When the bile is unable to leave the liver through the bile ducts, the liver becomes damaged and many vital body functions are affected.

The cause of biliary atresia is not known. Some researchers and physicians believe that babies are born with biliary atresia, implying the problem with the bile ducts occurred during pregnancy while the liver was developing. Others believe that the disease begins after birth, and may be caused by exposure to infections or exposures to toxic substances.

Biliary atresia does not seem to be linked to medications the mother took, illnesses the mother had, or anything else the mother did during her pregnancy. Currently, there is not a genetic link known for biliary atresia.

The disease is unlikely to occur more than once in a family.

Biliary atresia is the most common cause of chronic liver disease in neonates.

Biliary atresia occurs once in every 15,000 births.

Asian populations are most frequently affected. African Americans are affected approximately twice as much as Caucasians.

Biliary atresia causes liver damage and affects numerous important processes that allow the body to function normally. It is a life-threatening disease and is fatal without treatment.

Infants with biliary atresia usually appear healthy at birth. Most often, symptoms develop between two weeks to two months of life, and may include:

Jaundice

Dark urine

Light colored stools

Distended abdomen

Weight loss

Jaundice is a yellow discoloration of the skin and whites of the eyes due to an abnormally high level of bilirubin (bile pigment) in the bloodstream, which is then excreted through the kidneys. High levels of bilirubin may be attributed to inflammation or other abnormalities of the liver cells, or blockage of the bile ducts. Jaundice is usually the first sign, and sometimes the only sign, of liver disease.

Symptoms of biliary atresia may resemble other liver conditions or medical problems. Please consult your child’s physician for a diagnosis.

A physician or healthcare provider will examine your child and obtain a medical history. Several diagnostic procedures are done to help evaluate the problem and may include the following:

Blood tests

Liver enzymes - elevated levels of liver enzymes can alert physicians to liver damage or injury, since the enzymes leak from the liver into the bloodstream under these circumstances.

Bilirubin – bilirubin is produced by the liver and is excreted in the bile. Elevated levels of bilirubin often indicate an obstruction of bile flow or a defect in the processing of bile by the liver.

Albumin and total protein Below-normal levels of proteins made by the liver are associated with many chronic liver disorders.

Clotting studies, such as prothrombin time (PT) and partial thromboplastin time (PTT) Tests that measure the time it takes for blood to clot. Blood clotting requires vitamin K and proteins made by the liver.

Viral studies – including hepatitis and HIV Checking for viruses in the bloodstream can help determine the cause of the liver problems.

Blood culture – Checking for bacterial infection in the bloodstream that can affect the liver may be used to diagnose biliary atresia. Also imaging tests:

Abdominal ultrasound – a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view the liver, gallbladder, and bile ducts.

Hepatobiliary (HIDA) scan – a low radioactive isotope (technetium) is injected into the child’s vein. The liver and intestine are scanned by a nuclear medicine machine. If the isotope passes through the liver into the intestine, the bile ducts are open and the child does not have biliary atresia.

The test that gives the most definitive diagnosis is a liver biopsy. A tissue sample is taken from your child’s liver and examined for abnormalities, allowing biliary atresia to be distinguished from other liver problems.

Specific treatment for biliary atresia will be determined by your child’s physician based on the following:

The opinion of the physicians involved in the child’s care
your opinion and preference.

Biliary atresia is an irreversible problem. There are no medications that can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Until that happens, biliary atresia will not be curable. However, two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life. Your child’s physician can help determine whether either of these operations are an option.

Kasai portoenterostomy , this operation connects the bile drainage from the liver directly to the intestinal tract. It is most successful when done before an infant is 8 weeks old. The Kasai procedure is helpful because it can allow a child to grow and remain in fairly good health for several years. Eventually, cholestasis (backup of bile in the liver) will occur, causing liver damage. Up to 80 percent of children who undergo the Kasai portoenterostomy will eventually need to have a liver transplant.

Liver transplant - a liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. The new liver can be either:

A whole liver, received from a child who has died.

Part of a liver, received from a child who has died or
part of a liver, received from a relative or other person whose tissue types match the child’s tissue type.

After surgery, the new liver begins functioning and the child’s health often improves quickly. After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body’s normal protective mechanisms that helps fight against invasion of viruses, tumors, and other foreign substances. Anti-rejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ. Frequent contact with the physicians and other members of the transplant team is crucial after a liver transplant.

Before your child has either one of these operations, nutrition may be a problem. With biliary atresia, not enough bile reaches the intestine to assist with the digestion of fats in the diet. Protein deficiencies may occur due to liver damage. Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because of a faster metabolism.

Your physician may recommend that a pediatric nutritionist make recommendations regarding your child’s diet. Nutritional guidelines may include the following:

MCT (medium-chain triglyceride) oil or infant formulas with MCT (Portagen® or Pregestimil®) may be recommended to add extra calories to help your child grow. Medium-chain triglycerides are more easily digested without bile than other types of fats. MCT oil can be added to foods and liquids that your child eats.
Provide your child with high-calorie liquid feedings, as directed by your child’s physician. Some children with liver disease become too sick to eat normally. In this case, your physician may recommend that your child have liquid feedings given to help meet his/her body’s requirements. These feedings are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus, and into the stomach. A high-calorie liquid can be given through the tube to supplement your child’s diet if he/she is able to eat only small amounts of food, or to replace meals if your child is too sick to eat.

After surgery, your child’s digestion may return to normal, or you may still need to give extra vitamins and/or work with your child’s diet. Please consult your child’s physician for recommendations.
Many factors affect the long-term outlook for these children.

Some of them include:

The extent of bile duct damage.

The age at which either a Kasai portoenterostomy or liver transplant is done.

The extent of liver damage that has occurred.

The overall health of your child.

Over 65 percent of children who have the Kasai portoenterostomy will eventually require a liver transplant. After liver transplant, the child’s health will usually improve; however, a rigorous medical regimen must be followed.

Rubeola, also called 10-day measles, red measles, or measles, is a viral illness that results in a viral exanthem. Exanthem is another name for a rash or skin eruption. Rubeola has a distinct rash that helps aid in the diagnosis. It is spread from one child to another through direct contact with discharge from the nose and throat. Sometimes, it is spread through air-borne droplets from an infected child. This is a very contagious disease that usually consists of a rash, fever, and cough.

Measles virus, the cause of measles, is classified as a Morbillivirus. It is mostly seen in the winter and spring. Rubeola is preventable by proper immunization with the measles vaccine.
It may take between eight to 12 days for a child to develop symptoms of rubeola after being exposed to the disease. It is important to know that a child is contagious one to two days before the onset of signs and symptoms and three to five days after the rash develops. Therefore, children may be contagious before they even know they have the disease.

During the early phase of the disease (which lasts between one to four days), symptoms usually resemble those of an upper respiratory infection.

The following are the common symptoms of rubeola. However, each child may experience symptoms differently.
Symptoms may include:

Hacking cough

Redness and irritation of the eyes

Fever

Small red spots with white centers appear on the inside of the cheek (usually occur two days before the rash on the skin appears)

Rash – deep, red, flat rash that starts on the face and spreads down to the trunk, arms, and legs.

The rash starts as small distinct lesions, which then combines as one big rash. After three to four days, the rash will begin to clear leaving a brownish discoloration and skin peeling.

The most serious complications from rubeola include the following:

Ear infections

Pneumonia

Croup

Inflammation of the brain

The symptoms of rubeola may resemble other skin conditions or medical problems. Always consult your child’s physician for a diagnosis.

Rubeola is usually diagnosed based on a complete medical history and physical examination of your child. The lesions of rubeola are unique, and usually allow for a diagnosis simply on physical examination.

In addition, your child’s physician may order blood or urine tests to confirm the diagnosis.
Specific treatment for rubeola will be determined by your physician based on:

Your child’s age, overall health, and medical history
extent of the disease.

Aspirin and the Risk of Reye Syndrome in Children:
Do not give aspirin to a child without first contacting the child’s physician. Aspirin, when given as treatment for children, has been associated with Reye syndrome, a potentially serious or deadly disorder in children. Therefore, pediatricians and other healthcare providers recommend that aspirin (or any medication that contains aspirin) not be used to treat any viral illnesses in children.

The goal of treatment for rubeola is to help prevent the disease, or decrease the severity of the symptoms. Since it is a viral infection, there is no cure for rubeola. Treatment may include:

If your child was exposed and has not been immunized, your child’s physician may give the vaccine to the child within 72 hours to help prevent the disease.

Since the use of the rubeola (or measles) vaccine, the incidence of measles has decreased by 99 percent. About 5 percent of measles are due to vaccine failure. The measles vaccine is usually given in combination with the mumps and rubella vaccine. It is called the MMR. It is usually given when the child is 12 to 15 months old and then again between 4 to 6 years of age.

Other ways to prevent the spread of rubeola:
Children should not attend school or daycare for four days after the rash appears.
Assure all of your child’s contacts have been properly immunized.

Megaureter is an abnormality of one or both of the ureters of a child. Ureters are the two funnel-shaped tubes that carry urine from the kidneys to the bladder. A megaureter refers to an expanded or widened ureter that does not function normally. The size of a megaureter is usually greater than 7 millimeters in diameter.

Complications associated with megaureter include reverse flow of urine into the kidneys, and pooling of urine inside the ureter that does not drain. The pooling can cause a child to develop a urinary tract infection. In some children, complications from megaureter can cause kidney damage and failure.

A megaureter that is not associated with other problems occurs during fetal development. It occurs when a section of the ureter, which is normally a muscular layer of tissue, is replaced by stiff, fibrous tissue. In the absence of a muscular layer, normal peristalsis (worm-like movement of the ureter that propels urine towards the bladder) cannot occur.

Megaureter can occur alone, but usually occurs in combination with other disorders, such as prune belly syndrome.

The syndrome may occur in varying degrees, possibly causing blockage, and reverse flow of urine. However, each child may experience symptoms differently. The symptoms of a megaureter may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.

The severity of the problem often determines how a diagnosis is made. Often a megaureter is diagnosed by ultrasound while a woman is still pregnant. After birth, some children may have other problems that may suggest the presence of megaureter. Children who are diagnosed later often have developed urinary tract infections that require evaluation by a physician. This may prompt your child’s physician to perform further diagnostic tests, which may include the following:

Anatomy Megaureter

Intravenous pyelogram (IVP) – a diagnostic imaging technique which uses an x-ray to view the structures of the urinary tract. An intravenous contrast of dye is given so that the structures can be seen on film. An IVP also reveals the rate and path of urine flow through the urinary tract.

Voiding cystourethrogram (VCUG) – a specific x-ray that examines the urinary tract. A catheter (hollow tube) is placed in the urethra (tube that drains urine from the bladder to the outside of the body) and the bladder is filled with a liquid dye. X-ray images will be taken as the bladder fills and empties. The images will show if there is any reverse flow of urine into the ureters and kidneys.

Abdominal ultrasound – a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

Diuretic renal scan – a diagnostic nuclear imaging technique that is conducted by injecting a radioactive fluid into the vein. The radioactive material is then carried to the kidneys where it gives off signals that can be picked up by cameras. Midway during the procedure a diuretic medication is given to speed up urine flow through the kidneys. This helps detect any area of blockage in the urinary tract.

Specific treatment for megaureter will be determined by your child’s physician based on:

Your child’s age, overall health, and medical history
the extent of the disease

Your child’s tolerance for specific medications, procedures, or therapies
expectations for the course of the disease.

Your opinion or preference

Your child may require antibiotic therapy as a precaution to prevent future urinary tract infections.
In some cases, medical intervention is not required because the megaureter will resolve on its own over time. If there is a blockage of the urinary tract, however, a megaureter may require surgical intervention. The surgical procedure involves removing the section of the ureter that is abnormal, reducing it, and reconnecting the ureter.

Over half of all newborns develop some amount of jaundice, a yellow coloring in their skin, during the first week. This is usually a temporary condition, but may be a more serious sign of another illness. Jaundice is caused by the breakdown of red blood cells. As the old cells are broken down, hemoglobin is changed into bilirubin and removed by the liver. The build-up of bilirubin in the blood is called hyperbilirubinemia. Because bilirubin has a pigment, or coloring, it causes a yellowing of the baby’s skin and tissues. As liver function matures, the jaundice goes away. A premature infant is more likely to develop jaundice. The yellow tint to the skin can often be seen by gently pressing on the baby’s forehead or chest and watching the color return.
There are several types of jaundice:

Physiologic jaundice – Physiologic jaundice occurs as a “normal” response to the baby’s limited ability to excrete bilirubin in the first days of life.

Breast Milk Jaundice- About 2 percent of breastfed babies develop jaundice after the first week. Some develop breast milk jaundice in the first week due to low calorie intake or dehydration.

Hemolysis Jaundice– May occur with the breakdown of red blood cells due to hemolytic disease of the newborn (Rh disease), having too many red blood cells, or bleeding.

Jaundice related to inadequate liver function Jaundice may be related to inadequate liver function due to infection or other factors.

Although low levels of bilirubin are not usually a concern, large amounts can circulate to tissues in the brain and may cause seizures and brain damage. This is a condition called kernicterus.

The following are the most common symptoms of jaundice. However, each baby may experience symptoms differently.

Symptoms may include:

Yellow coloring of the baby’s skin – usually beginning on the face and moving down the body.

Poor feeding or lethargy

Symptoms of jaundice may resemble other conditions or medical problems. Always consult your baby’s physician for a diagnosis.

The timing of the appearance of jaundice helps with the diagnosis. Jaundice appearing in the first 24 hours is quite serious and usually requires immediate treatment. When jaundice appears on the second or third day, it is usually “physiologic.” However, it can be a more serious type of jaundice. When jaundice appears on the third day to the first week, it may be due to an infection. Later appearance of jaundice, in the second week, is often related to breast milk feedings, but may have other causes.

Laboratory testing for hyperbilirubinemia may include:

Direct and indirect bilirubin levels – These levels reflect whether the bilirubin is bound with other substances by the liver so that it can be excreted (direct), or is circulating in the blood circulation (indirect).

Red blood cell counts

Blood type and testing for Rh incompatibility (Coomb’s test)

Specific treatment for jaundice will be determined by your baby’s physician based on:

Your opinion or preference- This depends on many factors, including the cause of the jaundice and the level of bilirubin. The goal is to keep the level of bilirubin from increasing to dangerous levels.

Treatment may include:

Phototherapy– Because bilirubin absorbs light, jaundice and increased bilirubin levels usually decrease when the baby is exposed to special blue spectrum lights. Phototherapy may take several hours to begin working and it is used throughout the day and night. The baby’s position is changed to allow all of the skin to be exposed to the light. The baby’s eyes must be protected and the temperature monitored during phototherapy. Blood levels of bilirubin are checked to monitor if the phototherapy is working. Use of a fiberoptic blanket Another form of phototherapy is a fiberoptic blanket placed under the baby. This may be used alone or in combination with regular phototherapy.

Exchange transfusion – May be used to replace the baby’s damaged blood with fresh blood. This helps increase the red blood cell count and lower the levels of bilirubin. An exchange transfusion is done by alternating giving and withdrawing blood in small amounts through a vein or artery. Exchange transfusions may need to be repeated if the bilirubin levels remain high.

Discontinued breast feeding - Treatment of breast milk jaundice often requires stopping the breastfeeding for one to two days. Giving the baby formula often helps lower the bilirubin levels. Breastfeeding can then be resumed.

Treatment of underlying conditions - Treating any underlying cause of hyperbilirubinemia, such as infection.

While jaundice cannot be totally prevented, early recognition and treatment are important in preventing bilirubin levels from rising to dangerous levels. If your baby’s color is turning more yellow, promptly call your baby’s physician.

Prune Belly Syndrome is also known as Triad Syndrome or Eagle-Barrett syndrome. It is characterized by a triad of abnormalities that include the following:

Absence of abdominal muscles

Undescended testicles – a condition seen in newborns whereby one (or both) of the male testes has not passed down into the scrotal sac.

An abnormal, expanded bladder and problems in the upper urinary tract, which may include the bladder, ureters, and kidneys.

Because of the substantial involvement of the urinary tract, children with prune belly syndrome are usually unable to completely empty their bladders and have serious bladder, ureter, and kidney impairment.

A child with prune belly syndrome may also have other birth defects. Most commonly, these defects involve the skeletal system, intestines, and heart. Girls may have defects in their external genitalia, as well.
Some infants who have prune belly syndrome may be stillborn or die within a few months of birth.

Prune belly syndrome is an uncommon birth defect occurring in about one in 30,000 to 40,000 births. In 95 percent of the cases, it occurs in males. It may occur if there is a urethral obstruction during fetal development preventing any urine from flowing through the urinary tract. The urethra is the tube that drains urine from the bladder to the outside of the body for elimination. If there is obstruction, urine can reverse flow and cause an expanded bladder.

The cause of prune belly syndrome is unknown, however, some cases have been reported in siblings, suggesting a genetic component.

The syndrome may occur in varying degrees from mild to severe. The following are the most common symptoms of prune belly syndrome. However, each child may experience symptoms differently.

Symptoms may include:

The abdomen may have a wrinkly appearance with multiple folds of skin.

An abdominal mass may sit above the pubic bones – this is a result of an expanded bladder.

The urinary tract organs may be easy to feel through the abdominal area.

An outline of the intestines may be visualized through the abdomen including visualization of peristalsis (the worm-like movements of the intestines shifting food forward.)

Absence of testes in the scrotum may be present in males.

There may be underdeveloped muscles of the abdomen preventing a child from sitting upright.

A child may experience frequent urinary tract infections (usually uncommon in children younger than 5 years and unlikely in boys at any age, unless an obstruction is present.)

The symptoms of prune belly syndrome may resemble other conditions and medical problems. Always consult your child’s physician for a diagnosis.

The severity of the defects often determines how a diagnosis is made. Often prune belly syndrome is diagnosed by fetal ultrasound while a woman is still pregnant. Because of the distinct abnormalities of prune belly syndrome, a physician is usually able to make a diagnosis after initial examination during the newborn period. A child who may not have the outward signs of prune belly syndrome may experience a urinary tract infection, which will prompt further testing by your child’s physician.

Additional diagnostic procedures may include the following:

Renal Ultrasound – a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

Voiding Cystourethrogram (VCUG) – a specific x-ray that examines the urinary tract. A catheter (hollow tube) is placed in the urethra (tube that drains urine from the bladder to the outside of the body) and the bladder is filled with a liquid dye. X-ray images will be taken as the bladder fills and empties. The images will show if there is any reverse flow of urine into the ureters and kidneys.

Intravenous Pyelogram (IVP) – a diagnostic imaging technique which uses an x-ray to view the structures of the urinary tract. An intravenous contrast of dye is given so that the structures can be seen on film. An IVP also reveals the rate and path of urine flow through the urinary tract.

Blood Tests (to determine how well the kidneys may be functioning.)

Specific treatment for prune belly syndrome will be determined by your child’s physician based upon:

Your child’s age, overall health, and medical history the extent of the disease.

Treatment for the syndrome depends on the severity of symptoms. If your child has mild prune belly syndrome, he/she may be maintained on precautionary antibiotic therapy to prevent upper and lower urinary tract infections.

Your child may be referred to an urologist (physician who specializes in disorders and care of the urinary tract and the male genital tract). Some children may require a vesicostomy (a small opening made in the bladder through the abdomen) to facilitate emptying the bladder of urine. Other children may need to undergo extensive surgical remodeling of the abdominal wall and urinary tract. In boys, a surgical procedure to advance the testes into the scrotum may be performed, called an orchiopexy. Despite available surgical interventions, some children may develop extensive kidney failure.

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